Myelofibrosis

Question 1

What is myelofibrosis?

Answer 1

Myelofibrosis is a reactive and reversible process common to many malignant and benign bone marrow disorders.

Question 2

What are the risk factors of myelofibrosis?

Answer 2

> Radiation exposure
Industrial solvents exposure
age >65 years

Question 3

What are the signs and symptoms of myelofibrosis?

Answer 3

> Constitutional symptoms (FLAWS, cachexia)
> hepatosplenomegaly
> Portal htn
> Pulmonary HTN
> Joint/ bone pain
> hearing loss
> bleeding
> infections

Question 4

What investigations do you do for myelofibrosis?

Answer 4

> FBC- anaemia (ab)normal cell counts
Smear- teardrop shaped RBCs presence of metamyelocytes, myelocytes, promyelocytes, myeloblasts and nucleated RBCs
Unable to aspirate marrow
Bone marrow biopsy- marrow fibrosis

Question 5

What is the epidemiology of myelofibrosis?

Answer 5

In Europe, annual incidence of PMF ranges from 0.1 to 1 per 100,000 people.